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Chronic myeloid leukaemia

Find everything you need to know about Chronic Myeloid Leukaemia, including causes, symptoms, diagnosis and treatment, with links to other useful resources.

Key Information

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What should I do?

If you think you have this condition you should see a doctor within 48 hours.

How is it diagnosed?

Your doctor might suspect chronic myeloid leukaemia based on your symptoms and physical examination findings. A routine blood test could also suggest leukaemia. However, for a firm diagnosis, your doctor will need to perform a bone marrow biopsy. During this procedure, a needle is inserted into your hip bone under local anesthesia and a small sample is collected for analysis.

What is the treatment?

The first treatment for chronic myeloid leukaemia is generally medication which reduces the number of white blood cells that your body produces. These medications are available as tablets that are taken daily.

If these types of medication are not available or your cancer has progressed to a more advanced stage, your doctor might recommend chemotherapy.

The only cure for this condition is a bone marrow or stem cell transplant, however this isn’t suitable for everyone due to the associated risks.

When to worry?

If you have any of the following symptoms then please see a doctor immediately:

What happens in chronic leukaemia

All of the blood cells in your body are produced by bone marrow. Bone marrow is a spongy material found inside the bones. It is important because it produces special cells called stem cells.

Stem cells are very useful because they have the ability to create other specialised cells that carry out important functions. The stem cells in bone marrow produce three important types of blood cells:

red blood cells - which carry oxygen around the body

white blood cells - which help fight infection

platelets - which help stop bleeding

In leukaemia, the cancer begins in the stem cells and causes them to produce more white blood cells than are needed. Over time, the cancerous white blood cells build up, disrupting the normal balance of cells in the blood.

This means that the body does not have enough red blood cells or platelets. This can cause symptoms of anaemia, such as tiredness, as well as increasing the likelihood of excessive bleeding.

How common is chronic myeloid leukaemia?

Chronic leukaemia is an uncommon type of cancer.

Chronic myeloid leukaemia can affect people of any age, but it is more common in people aged 40-60. There is no evidence that it runs in families.

Outlook

The outlook for chronic myeloid leukaemia depends to a large extent on how well a person responds to medication.

Most patients (60-65%) do really well on imatinib tablets, which are taken every day for life.

For those who don't do well on imatinib, about half respond to one of the alternative drugs (nilotinib or dasatinib or bosutinib).

Symptoms

In its early stages, chronic myeloid leukaemia usually causes no noticeable symptoms.

When symptoms do develop, they typically include:

tiredness

loss of appetite

weight loss

a feeling of bloating

Chronic myeloid leukaemia can also cause swelling in your spleen (an organ that helps to filter impurities from your blood). This can cause a lump to appear on the left side of your abdomen, which may be painful when touched. A swollen spleen can also put pressure on your stomach, causing a lack of appetite and indigestion.

The symptoms of chronic myeloid leukaemia in its advanced stage will be much more noticeable and troublesome. They include:

severe fatigue

bone pain

night sweats

fever

easily bruised skin

Causes

Chronic myeloid leukaemia begins with an alteration to the structure of the DNA found in the stem cells responsible for producing white blood cells. This is known as a genetic mutation.

The DNA provides the cells with a basic set of instructions, such as when to grow and reproduce. The mutation in the DNA changes these instructions so that stem cells produce more white blood cells than are needed.

The white blood cells are also produced when they are still immature, so they do not have the infection-fighting properties of healthy 'adult' white blood cells.

Eventually, as the number of immature cells (blast cells) increases, it leads to a corresponding decrease in red blood cells and platelets. This reduction in other types of healthy blood cells causes many of the symptoms of chronic leukaemia.

Possible triggers for chronic leukaemia

What triggers the development of chronic leukaemia and causes the initial mutation in stem cells is unknown. The one proven risk factor is exposure to radiation.

However, radiation is only a significant risk if the levels are extremely high, such as those recorded after an atomic bomb explodes, or those released after a nuclear reactor accident, such as the one at Chernobyl.

Benzene

There is limited evidence that prolonged exposure to the chemical benzene leads to an increased risk of chronic myeloid leukaemia. Benzene is found in petrol and is also used in the rubber industry, but there are strict controls to protect people from prolonged exposure.

Benzene is also found in cigarettes. However, it is thought that smoking is more of a risk factor in acute leukaemia than it is in chronic leukaemia.

Occupational risks

A number of occupations have been linked to an increase risk of chronic leukaemia, possibly due to exposure to certain substances such as pesticides or chemicals.

These occupations include:

all types of agricultural workers

people who are involved with rubber or plastic manufacture

tailors and dressmakers

cleaners

builder’s labourer

Diagnosis

Suspected cases of chronic myeloid leukaemia are usually first detected after a blood test, which has often been carried out to diagnose another unrelated condition.

A blood test that reveals abnormally high levels of white blood cells could be a sign of chronic leukaemia. If you have a blood test with abnormal results, you will be referred to a haematologist (a specialist in treating blood conditions) for further testing.

Bone marrow biopsy

To confirm a diagnosis of chronic leukaemia, the haematologist will take a small sample of your bone marrow to examine under a microscope. This procedure is known as a bone marrow biopsy. A bone marrow biopsy is usually carried out under a local anaesthetic.

The haematologist will numb an area of skin at the back of your hip bone, before using a needle to remove the bone marrow sample. The procedure is usually painless although you may experience some bruising and discomfort for a few days afterwards. The procedure takes around 15 minutes to complete and you should not have to stay in hospital overnight.

The bone marrow sample will be checked to see if there are cancerous cells. If there are, the biopsy will also be able to help determine which type of chronic leukaemia is present.

Further tests

There are a number of additional tests that can be used to help reveal more information about the progress and extent of the leukaemia. These can also provide an insight into how the leukaemia should be treated. These are outlined below.

Cytogenetic testing

Cytogenetic testing involves identifying the genetic make-up of the cancerous cells. There are a number of specific genetic variations that can occur during leukaemia and knowing what these variations are can have an important impact on treatment.

For example, 90% of people with chronic myeloid leukaemia have the Philadelphia chromosome. People who have this chromosome are known to respond well to a medicine called imatinib.

Treatment

Imatinib tablets are usually given as soon as you have been diagnosed with chronic myeloid leukaemia, to slow its progression. These tablets are taken every day for life, and most patients do really well on them.

The aim of treatment is to achieve the following:

by 3 months, correct the blood count

by 12 months, clear the bone marrow of cells containing the Philadelphia chromosome (see Causes page for information on this)

by 18 months, get to a stage where the leukaemia can only be detected by a very sensitive molecular test (molecular remission)

Chemotherapy is usually offered if the cancer reaches an advanced stage.

These treatments are explained below.

Treating early-stage chronic myeloid leukaemia

Imatinib

A medicine called imatinib is the main treatment recommended for chronic myeloid leukaemia. It is usually given as soon as a diagnosis is made because the medicine is designed to slow the progression of the cancer and to prevent the condition reaching the accelerated or advanced phase.

Imatinib is a type of tyrosine kinase inhibitor. This means it blocks a protein called tyrosine kinase (tyrosine kinase helps to stimulate the growth of cancer cells). This reduces the production of abnormal white blood cells.

Imatinib is taken as a tablet. The side effects of imatinib are usually mild and should improve with time. They include:

nausea

vomiting

swelling in the face and lower legs

muscle cramps

rash

diarrhoea

Nilotinib

It is estimated that 10-40% of people who take imatinib become resistant to its effects, so an alternative treatment is required.

Nilotinib works in a similar way to imatinib in that it blocks the effects of proteins that help stimulate the growth of cancer cells.

Side effects of nilotinib include:

vomiting

abdominal pain

bone and joint pain

dry skin

loss of appetite

hair loss

insomnia

night sweats

dizziness

tingling or numbness

If the side effects become particularly troublesome, temporarily stopping the treatment usually helps to bring them under control. Treatment can then be resumed, possibly using a lower dose of medication.

Treating advanced chronic myeloid leukaemia

Chemotherapy

Once chronic myeloid leukaemia has progressed to a more advanced stage, chemotherapy is the next treatment.

Chemotherapy tablets are usually used first because they have fewer and milder side effects than chemotherapy injections. Side effects include:

tiredness

skin rash

increased vulnerability to infection

Chemotherapy can weaken your immune system, which helps protect you against infection. This is known as being immunocompromised. See chronic leukaemia – complications for more information about this.

If your symptoms persist or get worse, chemotherapy injections (intravenous chemotherapy) will need to be used. Intravenous chemotherapy causes more side effects than chemotherapy tablets and they tend to be more severe.

Side effects include:

nausea

vomiting

tiredness

hair loss

infertility

These side effects should resolve after your treatment has finished, although there is a risk that infertility could be permanent.

Bone marrow and stem cell transplants

A bone marrow transplant can offer a cure for chronic leukaemia, although it is only suitable and necessary for some patients.

Before transplantation can take place, the person receiving the transplant has to have aggressive, high-dose chemotherapy and radiotherapy to destroy any cancerous cells in their body.

This can put enormous strain on the body and can cause significant side effects and potential complications. Transplantations have better outcomes if the donor has the same tissue type as the person who is receiving the donation. The best candidate to provide a donation is usually a brother or sister with the same tissue type.

Due to these issues, transplantations are usually only successful when they are carried out in children and young people, or older people in good health, and there is a suitable brother or sister who can provide a donation.

In most cases of chronic leukaemia, the potential risks of transplantation far outweigh any benefit. For example, the chances of an elderly person with advanced chronic leukaemia surviving a bone marrow transplant can be as low as one in five.

However, your specific circumstances may mean that the benefits of treatment outweigh the risks.

Complications

Being compromised (having a weakened immune system) is a possible complication for some patients with chronic leukaemia.

There are two reasons for this:

the lack of healthy white blood cells means that your immune system is less able to fight infection

many of the medicines used to treat chronic leukaemia can weaken the immune system

This means that you are more vulnerable to developing an infection, and that any infection you have has an increased potential to cause serious complications.

You may be advised to take regular doses of antibiotics to prevent infections occurring. You should immediately report any possible symptoms of an infection to your doctor or care team because prompt treatment may be required to prevent serious complications.

Symptoms of infection include:

high temperature (fever) of 38C (101.4F) or above

headache

aching muscles

diarrhoea

tiredness

Avoid contact with anyone who is known to have an infection, even if it is a type of infection that you were previously immune to, such as chickenpox or measles. This is because your previous immunity to these conditions will probably be suppressed (lowered).

While it is important to go outside on a regular basis, both for exercise and for your psychological wellbeing, avoid visiting crowded places and using public transport during rush hour.

Also ensure that all of your vaccinations are up-to-date. Your doctor or care team will be able to advise you about this. You will be unable to have any vaccine that contains activated particles of viruses of bacteria such as:

the mumps, measles and rubella (MMR) vaccine

the polio vaccine

the oral typhoid vaccine

the BCG vaccine (used to vaccinate against tuberculosis)

the yellow fever vaccine

Psychological effects of chronic leukaemia

Receiving a diagnosis of chronic leukaemia can be very distressing, particularly if it is unlikely that your condition can be cured. At first, the news may be difficult to take in.

The situation can be made worse if you are confronted with the knowledge that even though your leukaemia may not currently be causing any symptoms, it could be a serious problem in later life. Having to wait many years to see how the leukaemia develops can be immensely stressful and can trigger feelings of anxiety and depression.

If you have been diagnosed with leukaemia, talking to a counsellor or psychiatrist (a doctor who specialises in treating mental health conditions) may help you to combat feelings of depression and anxiety. Antidepressants or medicines that help to reduce feelings of anxiety may also help you cope better with the condition.

You may find it useful to talk to other people who are living with leukaemia. Your doctor or multidisciplinary team may be able to provide you with details of local support groups.